|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Recently, the presence of a characteristic DNAJB1-PRKACA fusion gene has been associated with FLC.
|
31736218 |
2020 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
After establishing a novel FLC cell line that harbored the DNAJB1-PRKACA fusion, YAP1 was inhibited, which significantly reduced expression of known YAP1 target genes as well as cell growth and migration.
|
31669305 |
2020 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Fibrolamellar hepatocellular carcinoma (FLHCC) is driven by J-PKAcα, a kinase fusion chimera of the J domain of DnaJB1 with PKAcα, the catalytic subunit of protein kinase A (PKA).
|
30905674 |
2019 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Moreover, considering DNAJB1-PRKACA fusions lead to upregulated protein kinase activity and that this upregulated protein kinase activity has a significant role in tumorigenesis of fibrolamellar hepatocellular carcinoma, protein kinase inhibition could have therapeutic potential in the treatment of these pancreatobiliary neoplasms as well, once a suitable drug is developed.
|
31676785 |
2019 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Conformational Landscape of the PRKACA-DNAJB1 Chimeric Kinase, the Driver for Fibrolamellar Hepatocellular Carcinoma.
|
29335433 |
2018 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
By analyzing RNA-sequencing data from The Cancer Genome Atlas (TCGA) for >9,100 tumors across ~30 cancer types, we show that the DNAJB1-PRKACA fusion is specific to FLCs.
|
28304380 |
2017 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
The tumors contained the Dnajb1-Prkaca gene fusion and had histologic and cytologic features of human FL-HCCs: large polygonal cells with granular, eosinophilic, and mitochondria-rich cytoplasm, prominent nucleoli, and markers of hepatocytes and cholangiocytes.
|
28923495 |
2017 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Our study validates the <i>DNAJB1-PRKACA</i> fusion kinase as an oncogenic driver and candidate drug target for FL-HCC, and establishes a practical model for preclinical studies to identify strategies to treat this disease.
|
29162699 |
2017 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
This review summarizes recent advancements in FL-HCC pathogenesis and characteristics of the HSP40-PKA C protein.
|
26990031 |
2016 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
A consistent mutation in FL-HCCs leads to fusion of the genes encoding a heat shock protein (DNAJB1) and the catalytic subunit of protein kinase A (PRKACA).
|
27027723 |
2016 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Fibrolamellar carcinoma is characterized by a recurrent DNAJB1-PRKACA chimeric transcript.
|
26259677 |
2016 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
By using 78 clinically annotated FLC samples, we performed whole-transcriptome (n = 58), single-nucleotide polymorphism array (n = 41), and next-generation sequencing (n = 48) analyses; we also assessed the prevalence of the DNAJB1-PRKACA fusion transcript associated with this cancer (n = 73).
|
25557953 |
2015 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The lack of a second-hit mutation in the genomic landscape of fibrolamellar hepatocellular carcinoma makes the DNAJB1-PRKACA fusion protein the best target for diagnostic and therapeutic advancements.
|
25605237 |
2015 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
RT-PCR was successful in 92% of tested fibrolamellar carcinoma cases (24/26) and the DNAJB1-PRKACA fusion transcript was found in all fibrolamellar carcinomas but not in other tumor types.
|
25698061 |
2015 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Fibrolamellar hepatocellular carcinoma (FLHCC) tumors all carry a deletion of ∼ 400 kb in chromosome 19, resulting in a fusion of the genes for the heat shock protein, DNAJ (Hsp40) homolog, subfamily B, member 1, DNAJB1, and the catalytic subunit of protein kinase A, PRKACA.
|
26489647 |
2015 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
FusionGene
|
disease |
ORPHANET |
Evidence supporting the presence of the DNAJB1-PRKACA chimeric transcript in 100% of the FL-HCCs examined (15/15) suggests that this genetic alteration contributes to tumor pathogenesis.
|
24578576 |
2014 |
|
Fibrolamellar Hepatocellular Carcinoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Evidence supporting the presence of the DNAJB1-PRKACA chimeric transcript in 100% of the FL-HCCs examined (15/15) suggests that this genetic alteration contributes to tumor pathogenesis.
|
24578576 |
2014 |
|
Bipolar Disorder
|
0.320 |
AlteredExpression
|
disease |
BEFREE |
Expression of HSPF1 and LIM in the lymphoblastoid cells derived from patients with bipolar disorder and schizophrenia.
|
15362566 |
2004 |
|
Bipolar Disorder
|
0.320 |
Biomarker
|
disease |
PSYGENET |
In addition, we found the altered expression of LIM and HSPF1 both in the brains and lymphoblastoid cells in bipolar disorder.
|
14743183 |
2004 |
|
Bipolar Disorder
|
0.320 |
Biomarker
|
disease |
PSYGENET |
Expression of HSPF1 and LIM in the lymphoblastoid cells derived from patients with bipolar disorder and schizophrenia.
|
15362566 |
2004 |
|
Bipolar Disorder
|
0.320 |
AlteredExpression
|
disease |
LHGDN |
In addition, we found the altered expression of LIM and HSPF1 both in the brains and lymphoblastoid cells in bipolar disorder.
|
14743183 |
2004 |
|
Neoplasms
|
0.080 |
AlteredExpression
|
group |
BEFREE |
Moreover, considering DNAJB1-PRKACA fusions lead to upregulated protein kinase activity and that this upregulated protein kinase activity has a significant role in tumorigenesis of fibrolamellar hepatocellular carcinoma, protein kinase inhibition could have therapeutic potential in the treatment of these pancreatobiliary neoplasms as well, once a suitable drug is developed.
|
31676785 |
2019 |
|
Neoplasms
|
0.080 |
Biomarker
|
group |
BEFREE |
Using CRISPR/Cas9 technology, we found generation of the Dnajb1-Prkaca fusion gene in wild-type mice to be sufficient to initiate formation of tumors that have many features of human FL-HCC.
|
28923495 |
2017 |
|
Neoplasms
|
0.080 |
Biomarker
|
group |
BEFREE |
HLJ1 (DNAJB4) belongs to the DNAJ (HSP40) family of HSPs and is regarded as a tumor suppressor gene in lung, colon, and gastric cancers.
|
28481734 |
2017 |
|
Neoplasms
|
0.080 |
AlteredExpression
|
group |
BEFREE |
DNAJB1-PRKACA was expressed 10-fold higher than the wild-type PRKACA transcript, resulting in overexpression of the mutant protein in tumors.
|
27027723 |
2016 |